Chest physiotherapy
is an integral part of the management of CF. The aims are to reduce airway
obstruction by improving the clearance of secretions, to reduce the severity
of the infection by clearing infected material, and to maintain optimal
respiratory function and exercise tolerance (Cystic Fibrosis Trust, 2002).
Treatment is continually monitored and modified according to the individual’s
requirements.

Breathing control
is used between other techniques to allow relaxation.

Thoracic (chest)
expansion exercises with the emphasis on inspiration, expiration being
quiet and relaxed.

The forced expiration
technique or huff is used to mobilise and clear secretions. One or
two forced expirations are combined with a period of breathing control.
A huff from high lung volume (when a breath has been taken in) will clear
secretions from the upper airways and a huff from mid to low lung volume
will clear secretions from the lower more peripheral airways.

The Active Cycle of
Breathing Techniques (ACBT) are not a rigid treatment method and can be
modified to suit all ages and individual needs. It may be used in conjunction
with other airway clearance techniques as required.

Postural drainage

The aim of postural
drainage is to allow gravity to assist the drainage of respiratory secretions.
There are 11 different positions which are based on the anatomy of the
bronchial tree and are aimed at draining particular lobes or lung segments.
The right lung is divided into three lobes (upper, middle and lower) while
the left lung has only two lobes (upper and lower). Postural drainage
may be used in conjunction with other techniques, e.g. ACBT, positive
expiratory pressure (PEP) and percussion. It involves positioning to allow
gravity to assist drainage of secretions based on the bronchial tree anatomy.
Patients require an individual regimen of positions to manage their airway
clearance. This may be altered with disease progression or changing symptoms.
Positions can be modified if poorly tolerated or inconvenient.

Effective postural
drainage equipment for home use is provided from voluntary contributions
to the CF Unit funds.

Figure
1: Right upper lung (Click on picture to split lung into
lobes)

Right
upper lobe

Apical
segment (1)

Posterior
segment (2)

Anterior
segment (3)

Right
middle lobe

Lateral
segment (4)

Medial
segment (5)

Right
lower lobe

Superior
segment (6)

Anterior
basal segment (7)

lateral
basal segment (8)

posterior
basal segment (9)

medial
basal segment (10)

Figure
2. Left lung (Click on picture to split lung into lobes)

Left
upper lobe (superior division)

Apico-posterior
segment (11,12)

Anterior
segment (13)

Left
upper lobe (lingular division)

Superior
segment (14)

Inferior
segment (15)

Left
lower lobe

Superior
segment (16)

Posterior
basal segment (17)

Lateral
basal segment (18)

Antero-medial basal
segment (19)

Percussion/Chest
clapping

This can be performed
with cupped hand(s) over the area being drained. It should be performed
for approximately 15-20 seconds with pauses for five seconds or longer
to minimise the risk of desaturation in patients with moderate or severe
lung disease (Pryor et al, 1990). Mechanical percussors have
not been shown to increase sputum clearance or lung function above that
achieved with conventional manual techniques (Pryor et al, 1981).

Percussion in short
bursts can be used with ACBT until independent effective treatment can
be performed by the individual. Self treatment is initially supervised
and this continues until the patient, carer and physiotherapist consider
the treatment is carried out effectively. At the time of respiratory exacerbations,
assisted treatments are usually preferable.

Positive expiratory
pressure ("PEP") devices

Positive expiratory
pressure is used to open up and recruit obstructed lung, allowing air
to move behind secretions and assist in mobilising them. Breathing out
against a slight resistance (10 to 20 cms of water) prevents the smaller
bronchial tubes from collapsing down and thus permits the continuing upward
movement of any secretions (Tyrell et al, 1986; Falk & Anderson,
1991). The technique also allows the patient more independence.

Positive expiratory
pressure devices are available as a mask or a mouthpiece, (figure 3),
the suitability of which is discussed with the physiotherapist. When assessing
patients suitable for a PEP device, one of the eight available resistances
(diameters from 1.5 to 5mm) is selected to produce an expiratory pressure
of between 10 and 20cm of water during the middle phase of expiration.
The resistance is checked regularly with a manometer to ensure the desired
pressure is maintained.

The treatment can
be performed in the sitting or postural drainage position. The technique
involves a number of breaths, with slightly active expiration, through
the mask or mouthpiece followed by a period of huffing, coughing and breathing
control. This cycle is repeated as required until maximum clearance of
secretions is achieved. It may be combined with other physiotherapy techniques.
Bubble PEP has developed from conventional PEP therapy and can be useful
in younger children giving the benefits of positive pressure together
with distraction focus and fun.

Most benefit is obtained
by patients who produce sputum and have obstructed airways, where premature
airway closure during expiration contributes to retained secretions. PEP
permits greater patient independence and is useful when conventional techniques
may be difficult. In patients with a previous pneumothorax or bullae evident
on the chest X-ray or CT imaging, caution should be
exercised. PEP should not be used in patients with perforated ear drums.

Astra PEP

Pari PEP

Figure
3. Positive expiratory pressure devices

Oscillatory
devices

The Flutter VRP1 (Scandipharm
International, Powys, UK) is a small plastic device, which contains a
large ball bearing that repeatedly interrupts the outward flow of air.
This generates a controlled oscillating positive pressure, which mobilises
respiratory secretions. A Flutter session consists of about 10-15 breaths
followed by huffing and breathing control. This is repeated for 15 or
20 minutes depending on individual need.

Studies have been
conducted to assess the effect of the Flutter VRP1 with both positive
(Konstan et al, 1994; Homnick et al, 1998) and negative
(Pryor et al, 1994) results. The Flutter can be used as an adjunct
to other forms of physiotherapy or as a treatment in its own right (McIlwaine
et al, 1997). The Flutter resulted in greater improvement in
pulmonary function after one week in hospitalised patients when compared
to conventional chest physiotherapy, although there was no difference
detectable after two weeks treatment (Gondor et al, 1999).

The Acapella (DHD
Healthcare, Wampsville, NY 13163, USA) is a vibratory PEP device where
the resistance can be set, and is not position dependent. This means that,
unlike the Flutter, it can be used easily in postural drainage positions
without compromising patient comfort or efficacy of treatment.

Acapella
device

Flutter
device

Figure
4: Oscillatory devices

Autogenic
drainage

This term describes
a series of breathing exercises devised by the Belgian physiotherapist
Jean Chevaillier. The aim is to dislodge and collect mucus from the lungs
and then clear these secretions by breathing at various lung volumes (Chevaillier,
1984; Schoni, 1989).
There are three phases - the Unstick, Collect and Evacuate when breathing
at low, mid and high lung volumes to mobilise, collect and expectorate
secretions respectively. It should be taught by a person skilled in its
use and trained in the procedure. A modification of the original technique
is now used in Germany (David, 1991) and a recent trial has provided evidence
that the technique is of benefit (Miller et al, 1995).

We have found autogenic
drainage a difficult technique to use in young children, but it may be
beneficial in the young adolescent and in adults who are productive of
sputum and can co-operate fully, demonstrating effective breathing control.
This technique has been found to combat some associated problems of wheeze
and airway collapsibility and has been well received, particularly in
those individuals who suffer with uncontrolled coughing.

This is not an exhaustive
list of techniques, but is representative of those most commonly used.
All adjuncts to chest physiotherapy are assessed for effectiveness, suitability
and affordability by the specialist team.

Physical activity

Aerobic exercise is
beneficial for all patients with CF and should be encouraged. Most individuals
find exercise enjoyable with supervised exercise programmes improving
both cardiorespiratory fitness and morale, and leading to a higher degree
of perceived competence, improved self-esteem and quality of life. Exercise
should complement chest physiotherapy. The two treatment techniques combined
tend to increase sputum clearance (Andreasson et al, 1987; Bilton
et al, 1992; Baldwin et al, 1994; Webb et al, 1995).

Even in the very young
baby an increased level of activity is promoted. Parents are advised to
encourage the whole family to adopt an active lifestyle, so making exercise
part of normal family life. They are shown how to stimulate and encourage
age appropriate exercise from the time of diagnosis, and the resulting
benefits to the child are discussed.

The medical literature
is supportive of the importance of regular aerobic exercise to maintain
good lung function (Selvadurai et al, 2004). Anecdotally we have
observed very strong associations between those patients who regularly
participate in such exercise and those who maintain good lung function
and outcomes.

There are few activities
that the individual with CF cannot participate in following discussion
with the CF team. The following list gives examples of sports which may
cause issues:

• Scuba-diving
is not advised due to the increased risk of pneumothorax (BTS guidelines,
2003). Snorkelling is an enjoyable and acceptable alternative

• Horse-riding
is an acceptable activity but grooming or ‘mucking out’ should
be avoided due to the associated increased exposure to fungal spores

• Heavy weight
training should be avoided if the patient has an implantable venous access
device, as the catheter may become dislodged, and there is a risk of inguinal
hernia with all patients

High frequency
chest wall oscillation (The Vest TM)

This passive form
of airways clearance is used widely in the USA and some CF Centres in
this country are presently assessing it. Research has shown that The Vest
TM is as effective as conventional forms of chest physiotherapy (Braggion
et al, 1995), but it is a costly therapeutic option. The VestTM mobilises
pulmonary secretions by rapidly inflating and deflating, thereby compressing
the chest wall. This movement is thought to dislodge and thin the sputum
moving it into more proximal airways and aiding expectoration. We advocate
a more active and participative approach to airways clearance, although
certain patients may benefit from a trial of the device with a view to
longer term treatment.

Progression
of airways clearance techniques

It is important that
all patients are seen by an experienced physiotherapist with specialist
knowledge in the treatment of CF as soon as the diagnosis is confirmed.
If routine care is carried out at a local hospital it is essential to
have additional advice from the specialised team at a Regional CF Unit.

Following diagnosis
through neonatal screening the newborn infant will be seen regularly by
the physiotherapist. The parents will be taught how to identify increased
respiratory symptoms and when it is appropriate to increase the frequency
and nature of the treatment regimens. Passive manual techniques will be
demonstrated using positioning, percussion and vibrations. The families
will have the opportunity to practice these at home in order to become
proficient and confident in their use when respiratory symptoms occur
(Prasad & Main, 2006). The use of the head down position
in postural drainage is avoided at least for the first year to prevent
any possible negative effects from gastro-oesophageal reflux (GOR), a
common condition in infants with CF. Gastro-oesophageal reflux increases
the risk of aspiration of small amounts of food/fluid into the lungs.
GOR is associated with a more rapid deterioration in lung function and
may be exacerbated by head-down postural drainage (Button et al, 1997;
Button et al, 2004).

Treatment regimens
are designed for each individual. Active participation in airway clearance
is encouraged and initiated at appropriate ages. Blowing is the first
active participation in treatment and is introduced as soon as the child
is old enough. The forced expiratory technique (FET) and coughing are
introduced between the ages of three and five years. Between five and
seven years of age thoracic expansion exercises are incorporated to replace
the blowing. The treatment programme is regularly re-assessed and modified
to suit the individual patient.

The development from
this programme towards self-treatment is encouraged and stimulated. Some
children will be proficient in self-treatments from the age of 11 years,
while others prefer assisted treatments for longer. It is important that
adults with CF are educated in, and become responsible for, their own
chest care. They must recognise the signs of increased infection and contact
the hospital at these times for assessment and treatment with intravenous
antibiotics if necessary. Those who have not attended a specialist CF
Unit previously often require considerable education, explanation of the
importance of doing their chest physiotherapy treatment at home, and encouragement
to follow this programme on their own, now that they may no longer have
parental supervision. These patients require regular input, support and
monitoring from a physiotherapist experienced in dealing with and treating
adults who have CF.

Most adults with CF
produce sputum, and a daily session of appropriate chest physiotherapy
is encouraged with the type and timing adapted to patient needs and lifestyle.
Treatment may be increased to twice daily and more for patients producing
excess sputum, and during an exacerbation of their chest symptoms. Exercise
is actively encouraged for all patients, both for outpatients and whilst
in hospital, adjusting the exercise programme to each individual's requirement.

Conclusions

With modern treatment
many young patients with CF have no regular cough and produce no sputum
for many years. However, there is evidence that even in patients with
mild chest involvement regular daily physiotherapy helps to maintain respiratory
function. If the patient has a productive cough, physiotherapy should
be performed at least once daily, and more frequently with increasing
symptoms. We consider that regular monitoring of the patient’s and
family’s technique by a physiotherapist who is expert in the various
techniques used in the treatment of CF is essential (Morton et al,
1988; Worthington & Kelman, 1996). Lung damage does develop in
asymptomatic individuals and it is imperative that effective physiotherapy
techniques are routinely undertaken to clear secretions and protect the
airways from mucus plugging.

Sputum induction

It is uncommon for
young children to expectorate sputum. Older children and some adults may
only produce sputum with moderate to advanced disease. Early detection,
identification and treatment of lower respiratory tract infection are
an essential component of the management of CF. Cohort isolation and early
intensive treatment following initial isolation of P. aeruginosa,
results in a reduced incidence and prevalence of chronic P. aeruginosa
infection. Early infection can be eradicated.

Sputum induction with
nebulised hypertonic saline is safe and well tolerated. It can be delivered
in the clinic. Four mls of 6% or 7% saline is given via a standard nebuliser
10 minutes after 2.5mg of nebulised salbutamol. The patient is assessed
with chest auscultation, respiratory function tests and oxygen saturation
before and after the saline inhalation (Ho et al, 2004; De Boeck
et al, 2000).

In our study of sputum
induction in children with CF, a total of 43 tests were performed (median
age five years, range 1.2 to12 years). Specimens obtained by sputum induction
were significantly more likely to give additional microbiological information
than standard specimens, p=0.006 (Ho et al, 2004).

We advocate obtaining
an induced specimen when standard cough swab specimens do not provide
useful information in the following situations; if a patient has a new
cough that has not responded to empirical oral or intravenous antibiotics,
has new chest x-ray changes, or converts to P. aeruginosa antibody
positive.